With advancements in complex repairs in neonates with complicated congenital heart diseases, extracorporeal membrane oxygenation (ECMO) has been increasingly used as cardiac support. ECMO has also been increasingly used for low birth weight (LBW) or very low birth weight (VLBW) neonates. However, since prematurity and LBW are risk factors for ECMO, the appropriate indications for neonates with LBW, especially VLBW, are under dispute. We report a case of ECMO performed in a 1,360-g premature infant with VLBW due to cardiopulmonary bypass weaning failure after repairing infracardiac total anomalous pulmonary venous return.
Cardiopulmonary Bypass ; Dissent and Disputes ; Extracorporeal Membrane Oxygenation* ; Heart Diseases ; Humans ; Infant, Low Birth Weight ; Infant, Newborn* ; Infant, Premature ; Infant, Very Low Birth Weight ; Risk Factors ; Scimitar Syndrome* ; Weaning

A 50-year-old female patient with visual disturbances was referred for further evaluation of a heart murmur. Fundoscopy revealed a Roth spot in both eyes. A physical examination showed peripheral signs of infective endocarditis, including Osler nodes, Janeway lesions, and splinter hemorrhages. Our preoperative diagnosis was subacute bacterial endocarditis with severe aortic regurgitation. The patient underwent aortic valve replacement and was treated with intravenous antibiotics for 6 weeks postoperatively. The patient made a remarkable recovery and was discharged without complications. We report this case of subacute endocarditis with all 4 classic peripheral signs in a patient who presented with visual disturbance.
Anti-Bacterial Agents ; Aortic Valve ; Aortic Valve Insufficiency ; Diagnosis ; Endocarditis ; Endocarditis, Subacute Bacterial* ; Female ; Heart Murmurs ; Hemorrhage ; Humans ; Middle Aged ; Physical Examination

OBJECTIVE: The 2010 New American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for rheumatoid arthritis (RA) was raised to identify patients with early RA and replaced the 1987 ACR classification criteria. The aims of this study are to assess the availability of new classification criteria and to evaluate its potential limitation. METHODS: A total of 408 patients with newly diagnosed RA were included from 13 secondary or tertiary hospitals in South Korea. The symptom duration was less than 12 months before the diagnosis of RA. RA was defined as either 1987 ACR classification criteria or new 2010 ACR/EULAR criteria. We compared the full details of both classification criteria. RESULTS: The mean symptom duration was 5.1 months. The majority (76.2%) of the patients were female. Two hundred and seventy three patients (66.9%) fulfilled both of the 2010 and 1987 classification criteria. Forty-seven (14.7%) of the 320 patients fulfilling the 1987 criteria did not fulfill the new classification criteria. On the other hand, eighty-eight (24.4%) of the 361 patients fulfilling the 2010 ACR/EULAR classification criteria did not fulfill the 1987 ACR criteria. Thirty-six (55.4%) of the 65 patient with seronegative RA failed to meet the 2010 classification criteria. In case of seropositive RA (n=343), 85 additional patients (24.8%) could be diagnosed as RA using new classification criteria. CONCLUSION: The new 2010 ACR/EULAR classification criteria enable physicians to diagnose more patients with early RA via the help of serology. However, the sensitivity for the diagnosis of seronegative RA is projected to decrease.
Arthritis, Rheumatoid ; Female ; Hand ; Humans ; Republic of Korea ; Rheumatic Diseases ; Tertiary Care Centers

Cryptococcal meningitis is a rare complication of systemic lupus erythematosus (SLE). The nonspecific neurologic findings associated with this infection delays accurate diagnosis because initial neuropsychiatric manifestations of SLE are in instances indistinguishable from that of crytococcal meningitis. We report a case of cryptococcal meningitis presenting with unilateral sixth cranial nerve palsy in a male patient with SLE, which was successfully treated with antifungal agents.
Abducens Nerve Diseases/*etiology ; Adult ; Humans ; Lupus Erythematosus, Systemic/*complications ; Male ; Meningitis, Cryptococcal/*etiology

OBJECTIVE: To investigate the extraglandular manifestations and serological features of Korean patients with primary Sjogren's syndrome (pSS). METHODS: Clinical and laboratory data of 125 patients with pSS attending rheumatology clinic of Kangnam St. Mary's hospital, with a mean follow-up period of 64.8 months, were assessed retrospectively. RESULTS: Arthralgia was the most common extraglandular manifestation of pSS. Other extraglandular manifestations, such as myalgia, peripheral neuropathy, Raynaud's phenomenon, and hypothyroidism were frequently presented, but lymphoma was rare manifestation in Korean patients with pSS. Some extraglandular manifestations of pSS were associated with hypocomplementemia and antibodies against Ro, and La, and antinuclear antibody. CONCLUSION: The clinical manifestations and extraglandular manifestations of Korean patients with pSS patients were similar to other studies except low prevalence of lymphoma. Positivity of anti-Ro/SS-A, anti-La/SS-B antibodies and hypocomplementemia were closely associated with extraglandular manifestations in patients with pSS.
Antibodies ; Antibodies, Antinuclear ; Arthralgia ; Autoantibodies* ; Follow-Up Studies ; Humans ; Hypothyroidism ; Lymphoma ; Myalgia ; Peripheral Nervous System Diseases ; Prevalence ; Retrospective Studies ; Rheumatology ; Sjogren's Syndrome*

Objective: To investigate the effect of tumor necrosis factor (TNF)-alpha on the production of macrophage migration inhibitory factor (MIF), which might have important roles in the immune mediated inflammatory response of Behcet's syndrome. Methods: Sixty two patients with Behcet's syndrome and thirty healthy controls were included in this study. The concentrations of TNF-alpha in sera were determined by enzyme-linked immunosorbent assay (ELISA). Peripheral blood mononuclear cells (PBMCs) from eleven patients with Behcet's syndrome were cultured for 48 hours with various concentration of TNF-alpha. The concentrations of MIF in sera and culture supernatants were determined by ELISA. Results: Serum levels of TNF-alpha were significantly higher in patients with Behcet's syndrome than in healthy controls. TNF-alpha dose-dependently increased MIF production from PBMCs in patients with Behcet's syndrome. Serum levels of TNF-alpha tended to correlate with serum levels of MIF, although did not reach statistical significance. Conclusion: Upregulation of MIF production by increased levels of TNF-alpha in patients with Behcet's syndrome might be related to the pathogenesis of Behcet's syndrome.
Behcet Syndrome* ; Enzyme-Linked Immunosorbent Assay ; Humans ; Macrophages* ; Tumor Necrosis Factor-alpha* ; Up-Regulation*

OBJECTIVE: To investigate the clinical manifestation and prognostic factors of interstitial lung disease (ILD) in Korean patients with idiopathic inflammatory myopathies include with polymyositis (PM) and dermatomyositis (DM). METHODS: Clinical and laboratory data of 110 patients with PM/DM in our rheumatology clinic were investigated. Clinical data including history, medication, pulmonary function tests (PFT) findings, radiologic findings, and labaratory findings were obtained from medical records at the first diagnosis of ILD with PM/DM. ILD was diagnosed on the basis of the imaging abnormalities defined above on definite findings of chest X-rays and high resolution computed tomography (HRCT), restrictive changes on PFT with respiratory symptoms. During the course of treatment, we assessed chest radiograph and HRCT findings. RESULTS: Forty-two PM/DM patients (38.2%) developed ILD. Anti-extracellular nuclear antigen (ENA) antibody, anti-Jo-1 antibody and ground glass opacity in HRCT were significantly high in PM-ILD. However honeycoomb appearance (53% : 22%) and fibrosis (41% : 6%) in HRCT were significantly high in DM-ILD. Interest in aspects of prognosis including initial steroid treatment response in HRCT were favorable in PM-ILD. There were statistically significant association between normal level of CPK and usual interstitial pneumonia (UIP) pattern in HRCT in DM-ILD. Such cases had resistance to steroid therapy. Overall interval between steroid and immunosuppressant therapy was significantly shorter in those with DM-ILD. CONCLUSION: The clinical manifestations between PM-ILD and DM-ILD in Korean patients were not significant different from those of other populations. DM-ILD is more refractory to steroid treatment, expecting in poor prognosis compared with PM-ILD. So immediate intensive immunosuppressive therapy should be considered in DM-ILD.
Dermatomyositis ; Diagnosis ; Fibrosis ; Glass ; Humans ; Idiopathic Pulmonary Fibrosis ; Lung Diseases, Interstitial ; Lung* ; Medical Records ; Myositis* ; Polymyositis ; Prognosis ; Radiography, Thoracic ; Respiratory Function Tests ; Rheumatology ; Thorax

No Abstract available.
Ankle* ; Sarcoidosis* ; Tenosynovitis*

No abstract available.
Tenosynovitis*

OBJECTIVE: CD27 is a member of the tumor necrosis factor receptor (TNFR) superfamily and is expressed on T, B, and NK cells. The signaling via CD27 plays pivotal roles in T-T and T-B interaction. CD27 is a useful marker in assessing the number of circulation B cells and B cell subsets because it permits one step identification of the major B cell compartments, CD27- naive and CD27+ memory B cells as well as CD27high plasma cells. We have analyzed the mechanisms underlying the regulation of CD27 expression. METHODS: Isolation B cells and Raji cells were cultured with PMA. The levels of cell surface CD27 and CD 27 mRNA were analyzed by FACs staining and RT-PCR. Raji cells were cultured with phorbol 12-myristate 13-acetate (PMA), with or without pretreated shedding inhibitor BB3103 and TAPI-1. sCD27 was measured in culture supernatant by ELISA. Cell lysates were analyzed for PKC isotype activation by Western blot. We used PKC inhibitor Ly379196 and rottlen. RESULTS: Membrane expression of CD27 was down-regulated after PMA stimulation without cytotoxic effect in B cells. Furthermore, PMA treatment could directly reduce CD27 mRNA without intermediate protein synthesis in B cells. In contrast, PMA treatment induced soluble form of CD27 (sCD27), which was shed from the cell surface and was found in PMA treatment B cell culture supernatant. PMA-induced sCD27 proteins were decreased with shedding inhibitor BB3103 and TAPI-1. PMA-induced down regulation of CD27 expressions were quenched with protein kinase C (PKC) inhibitor Staurosporin, PKC-beta inhibitor rottlerin and PKC-delta inhibitor Ly379196. CONCLUSION: These data suggest that PMA-induced activation of PKC plays a crucial role in down-regulation of the expression of the CD27 and up-regulation of the shedding of the CD27 in human B cells.
B-Lymphocyte Subsets ; B-Lymphocytes* ; Blotting, Western ; Cell Culture Techniques ; Down-Regulation* ; Enzyme-Linked Immunosorbent Assay ; Humans ; Killer Cells, Natural ; Membranes* ; Memory ; Plasma Cells ; Protein Kinase C ; Protein Kinase C-delta* ; Protein Kinases* ; Receptors, Tumor Necrosis Factor ; RNA, Messenger ; Up-Regulation